OBJECTIVE To investigate the long-term outcomes of patients with combined primary sclerosing cholangitis/inflammatory bowel disease (PSC-IBD) undergoing both liver transplantation (LT) and total abdominal colectomy (TAC). SUMMARY BACKGROUND DATA The fraction of patients with PSC-IBD that require both LT and TAC is small, thereby limiting significant conclusions regarding long-term outcomes. METHODS Adult and pediatric patients from nine centers from the US IBD Surgery Collaborative who underwent staged LT and TAC for PSC-IBD were included. Long-term outcomes, including survival, were assessed. RESULTS Among 127 patients, 66 underwent TAC-before-LT, with a median time from TAC to LT of 7.9 yrs, while 61 underwent LT-before-TAC, with a median time from LT to TAC of 4.4 years. Median patient survival post TAC was significantly worse in those undergoing LT-before-TAC (16.0 yrs vs. 42.6 yrs, P=0.007), while post LT survival was not impacted by the order of TAC and LT (21.6 yrs vs. 22.0 yrs, P=0.81). Patients undergoing TAC for medically refractory disease had a higher incidence of recurrent PSC (rPSC) (P=0.02) and biliary complications (0.09) compared to those undergoing TAC for oncologic indications. Definitive TAC reconstruction with either end ileostomy or ileal-pouch anal anastomosis (IPAA) did not impact post-LT or post-TAC outcomes. CONCLUSIONS Long term survival in PSC-IBD was contingent upon progression to LT and was not impacted by the need for TAC. PSC-IBD patients undergoing TAC for medically refractory disease had a higher incidence of rPSC and biliary complications. The use of IPAA in PSC-IBD was a viable alternative to end ileostomy.

中文翻译：

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结肠切除术和肝移植治疗伴有原发性硬化性胆管炎的炎症性肠病的长期结果。


目的 调查合并原发性硬化性胆管炎/炎症性肠病 (PSC-IBD) 患者接受肝移植 (LT) 和全腹部结肠切除术 (TAC) 的长期结局。摘要背景数据 需要 LT 和 TAC 的 PSC-IBD 患者比例很小，因此限制了有关长期结果的重要结论。方法 纳入来自美国 IBD 外科合作组织 9 个中心、因 PSC-IBD 接受分期 LT 和 TAC 的成人和儿童患者。评估了长期结果，包括生存率。结果 127 例患者中，66 例接受 TAC-before-LT，从 TAC 到 LT 的中位时间为 7.9 年，61 例接受 LT-before-TAC，从 LT 到 TAC 的中位时间为 4.4 年。在 TAC 之前接受 LT 的患者中，TAC 后患者的中位生存期明显较差（16.0 岁 vs. 42.6 岁，P=0.007），而 LT 后生存期不受 TAC 和 LT 顺序的影响（21.6 岁 vs. 22.0 岁） ，P=0.81）。与因肿瘤适应症接受 TAC 的患者相比，因难治性疾病接受 TAC 的患者复发性 PSC (rPSC) (P=0.02) 和胆道并发症 (0.09) 的发生率较高。通过末端回肠造口术或回肠袋肛门吻合术 (IPAA) 进行的最终 TAC 重建不会影响 LT 后或 TAC 后的结果。结论 PSC-IBD 的长期生存取决于进展为 LT，并且不受 TAC 需求的影响。因难治性疾病而接受 TAC 的 PSC-IBD 患者 rPSC 和胆道并发症的发生率较高。在 PSC-IBD 中使用 IPAA 是末端回肠造口术的可行替代方案。